Diaphragm pacing and independent breathing in individuals with severe Pompe disease

• Główni autorzy: Cristina Liberati (Autor), Barry J. Byrne (Autor), David D. Fuller (Autor), Chasen Croft (Autor), Teresa Pitts (Autor), Jessica Ehrbar (Autor), Carmen Leon-Astudillo (Autor), Barbara K. Smith (Autor)
• Format: Książka
• Wydane: Frontiers Media S.A., 2023-07-01T00:00:00Z.
• Hasła przedmiotowe: article
• Dostęp online: Connect to this object online.

IntroductionPompe disease is an inherited disease characterized by a deficit in acid-α-glucosidase (GAA), an enzyme which degrades lysosomal glycogen. The phrenic-diaphragm motor system is affected preferentially, and respiratory failure often occurs despite GAA enzyme replacement therapy. We hypoth...